1,355 results match your criteria: "EEG in Common Epilepsy Syndromes"

CASPR2 antibody associated neurological syndromes in children.

Sci Rep

February 2023

Hunan Key Laboratory of Pediatric Emergency Medicine, Hunan Children's Hospital, The School of Pediatrics, Hengyang Medical School, University of South China, Changsha, Hunan, China.

To strengthen the understanding of the clinical features for CASPR2 neurological autoimmunity in children. A multicenter retrospective and prospective analysis of CASPR2 autoimmunity was conducted. Twenty-six patients were enrolled, including 25 with serum positivity and 3 with cerebrospinal fluid (CSF) positivity; 5 patients were co-positive with anti-NMDAR or anti-GABABR antibodies.

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Objectives: Despite the prevalence of cognitive symptoms in the idiopathic generalized epilepsies (IGEs), cognitive dysfunction in juvenile absence epilepsy (JAE), a common yet understudied IGE subtype, remains poorly understood. This descriptive study provides a novel, comprehensive characterization of cognitive functioning in a JAE sample and examines the relationship between cognition and 24-h epileptiform discharge load.

Method: Forty-four individuals diagnosed with JAE underwent cognitive assessment using Woodcock Johnson III Test of Cognitive Abilities with concurrent 24-h ambulatory EEG monitoring.

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Clinical and Electrophysiological Features Predicting Response to Antiseizure Medications in Juvenile Absence Epilepsy.

Neuropediatrics

February 2023

Bakırçay University, Çiğli Regional Education Hospital, Department of Pediatrics, Division of Pediatric Neurology, İzmir, Turkey.

Background: We aimed to evaluate the clinical findings and electroencephalographic (EEG) characteristics of patients with juvenile absence epilepsy (JAE) and to determine the factors that predict response to antiseizure medications (ASMs) in JAE.

Methods: We reviewed the medical records of 29 patients with JAE. The patients who were seizure-free during the last 12 months of their follow-up and who did not have generalized spike waves on their last EEG were considered as the treatment-responsive group, and the patients whose clinical seizures persisted during the last 12 months of their follow-up or who had generalized spike waves on their follow-up EEGs were considered as patients who did not respond to ASMs.

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Background  This study aims to examine the clinico-etiological profile of children with the first episode of a seizure and categorize seizure types based on age groups in a tertiary care hospital. Methodology  This was a prospective observational study conducted at a tertiary care medical institute in India over two years. Children (one month to 12 years of age) with the first episode of a seizure were included in the study population.

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Stereoelectroencephalography-based research on the value of drug-resistant temporal lobe epilepsy auras: A retrospective single-center study.

Epilepsy Behav

January 2023

Department of Neurosurgery, Anhui Provincial Hospital, WanNan Medical College, Wuhu, PR China; Department of Neurosurgery, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei 230001, PR China; Anhui Provincial Institute of Stereotactic Neurosurgery, 9 Lujiang Road, Hefei, Anhui Province 230001, PR China. Electronic address:

Purpose: To explore the localization value of drug-resistant temporal lobe epilepsy (TLE) aura for preoperative evaluation, based on stereoelectroencephalography (SEEG), and its prognostic value on the surgical outcome.

Methods: The data of patients with drug-resistant TLE who had SEEG electrodes implanted during preoperative evaluation at the First Affiliated Hospital of the University of Science and Technology of China (Hefei, China) were retrospectively analyzed. The patients were divided into aura-positive and aura-negative groups according to the presence of aura in seizures.

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Blinking in children is most frequently a functional and transient symptom. Nonetheless, sometimes it is the first clinical manifestation of a neurological disorder. The differential diagnosis between voluntary actions, tics and other neurological disorders among which seizures may be challenging and misdiagnosis is common.

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Epilepsy is a heterogeneous disorder characterized by spontaneous seizures and behavioral comorbidities. The underlying mechanisms of seizures and epilepsy across various syndromes lead to diverse clinical presentation and features. Similarly, animal models of epilepsy arise from numerous dissimilar inciting events.

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Lennox Gastaut Syndrome - A strategic shift in diagnosis over time?

Seizure

December 2022

Paediatric Neurology Department, Great Ormond Street Hospital, Great Ormond Street, London WC1N 3JH, UK.

Background: Lennox Gastaut Syndrome (LGS) is an epilepsy syndrome presenting in childhood, classically characterised by a triad of cognitive or developmental impairment, multiple seizure types and EEG features of slow-spike waves (SWW), with or without paroxysmal fast activity (PFA) in sleep. There is increasing scientific opinion in favour of a less rigid approach to LGS diagnosis and this clinical audit attempts to shed light on how the LGS diagnostic criteria used may have changed over time, in a large tertiary paediatric neurology unit (Great Ormond Street Hospital (GOSH), London, UK).

Methods: Electronic patient records were reviewed for patients with a diagnosis of LGS made at GOSH within two time periods, 2014-2017 and 2018-2021.

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To summarize the genetics and clinical phenotypes of epilepsy children with 2q24.3 microdeletion. All the patients with 2q24.

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Pattern and etiology of early childhood epilepsy: An Experience at a tertiary care University Center.

Neurosciences (Riyadh)

October 2022

From the Division of Pediatric Neurology (Kentab, Al Bulayhi, Hamad, Al Wadei, Bashiri), Department of Pediatrics, King Khalid University Hospital, King Saud University Medical City, and from the Department of Pediatrics (Kentab, Bashiri), College of Medicine, King Saud University, and from the Department of Pediatric Neurology (Al Wadei), National Neuroscience Institute, King Fahad Medical City, Riyadh, Kingdom of Saudi Arabia.

Objectives: To investigate seizure characteristics, types, and define the etiology of epilepsy in children aged ≤2 years using the 2017 ILAE classification.

Methods: A retrospective chart review was conducted at King Khalid University Hospital, King Saud University Medical City, Riyadh, Kingdom of Saudi Arabia for children below 2 years of age diagnosed with epilepsy, and on anti-seizure medications from January 2017 - December 2018. The collected data involved detailed information on the patients' seizure, electroclinical, neuroimaging, laboratory evaluations, and underlying etiology.

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Introduction: Temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS) is one of the most common drug-resistant epilepsy. Surgery is currently accepted as an effective and safe therapeutic approach compared to antiseizure medications (ASMs). The study aims to evaluate the effect of surgical treatment of TLE-HS on sleep profile and architecture by subjective and objective evaluation of sleep in basal condition after one month and one year.

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Epileptic spasms: A South African overview of aetiologies, interventions, and outcomes.

Dev Med Child Neurol

April 2023

Paediatric Neurology Division, Department of Paediatrics and Child Health, Red Cross War Memorial Children's Hospital, Neuroscience Institute, University of Cape Town, Cape Town, South Africa.

Aim: To better understand the aetiologies of epileptic spasms in infants, as well as the safety and efficacy of high dose corticosteroids in tuberculosis and human immunodeficiency virus (HIV) endemic resource-limited settings.

Method: This was a retrospective analysis of infants with epileptic spasms managed at the tertiary referral centres in the Western Cape, South Africa.

Results: Of 175 children with epileptic spasms, the median age at onset was 6 months (interquartile range 4-8 months).

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Obsessive-compulsive symptoms in ACTG1-associated Baraitser-Winter cerebrofrontofacial syndrome.

J Neural Transm (Vienna)

November 2022

Department of Psychiatry and Psychotherapy, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany.

Symptoms of obsessive-compulsive disorder (OCD) may rarely occur in the context of genetic syndromes. So far, an association between obsessive-compulsive symptoms (OCS) and ACTG1-associated Baraitser-Winter cerebrofrontofacial syndrome has not been described as yet. A thoroughly phenotyped patient with OCS and ACTG1-associated Baraitser-Winter cerebrofrontofacial syndrome is presented.

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Diagnostic and prognostic value of EEG patterns recorded on foramen ovale and epidural peg electrodes.

Clin Neurophysiol

November 2022

Epilepsy-Center Berlin-Brandenburg, Institute for Diagnostics of Epilepsy, Berlin, Germany; Epilepsy-Center Berlin-Brandenburg, Department of Neurology, Charité - Universitätsmedizin Berlin, Berlin, Germany.

Objective: To describe and assess the significance of EEG characteristics recorded during presurgical video-EEG monitoring (VEM) utilizing foramen ovale (FO) and epidural peg electrodes.

Methods: Seizure onset (SOP) and termination pattern morphology and regions, ipsilateral and contralateral latencies, seizure duration, and interictal spike counts were examined in 106 patients (412 seizures). An EEG feature-based logistic regression model predicting one-year post-surgical seizure freedom was assessed using a 5-fold nested cross-validation approach.

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Aim: To explore the phenotypic spectrum and refine the genotype-phenotype correlation of DYNC1H1-related epilepsy.

Method: The clinical data of 15 patients with epilepsy in our cohort and 50 patients with epilepsy from 24 published studies with the DYNC1H1 variants were evaluated.

Results: In our cohort, 13 variants were identified from 15 patients (seven males, eight females).

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Objective: To study the literature data and a series of our cases regarding the epilepsy clinic, electroencephalographic changes and other phenotypic features in X-linked intellectual disability (ID) caused by mutations.

Material And Methods: We analyzed the anamnesis of the disease, using medical records from different Russian medical organizations, as well as the results of the genealogical anamnesis, clinical, genetic, electroencephalographic (EEG) and neuroimaging (brain MRI ) examinations of 7 patients (5 girls and 2 boys aged 5 to 13 years) with a confirmed diagnosis of X-linked ID caused by mutations, in whom the clinical picture of the underlying disease was combined with epilepsy.

Results: The main common phenotypic features of patients with X-linked ID caused by the mutations are mental retardation, lack of phrasal speech, motor developmental delay, and dysmorphism.

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Behavior during 3-4 Hz spike-wave discharges (SWDs) in absence epilepsy can vary from obvious behavioral arrest to no detectible deficits. Knowing if behavior is impaired is crucial for clinical care but may be difficult to determine without specialized behavioral testing, often inaccessible in practice. We aimed to develop a pure electroencephalography (EEG)-based machine-learning method to predict SWD-related behavioral impairment.

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Case report: An EEG captured case of migralepsy/migraine aura-triggered seizures.

Front Neurol

August 2022

Department of Neurology, Virginia Tech Carilion, Roanoke, VA, United States.

Introduction: Migraine and epilepsy are common chronic neurological disorders presenting with paroxysmal attacks of transient cerebral dysfunction, followed by subsequent return to baseline between episodes. The term "migralepsy" has been proposed to define migraine-triggered epileptic seizures classified by the ICHD-III as a complication of migraine with an aura.

Case: A 55-year-old man with a 30-year history of migraine without aura presented with a new onset left parietal pain accompanied by visual disturbances occurring up to 20 times per day.

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Objective: To determine the contribution of genetic etiologies in epilepsy with photosensitivity.

Methods: A total of 35 epileptic patients with genetic photosensitivity from January 2019 to May 2021 were analyzed.

Results: Pathogenic variants were identified in 35 patients, including (7) (6), (3), (3), (2), (1), (1), (1), (1) (1), (1), (1), (1), (1), (1), 5q33.

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Semiology, EEG, and neuroimaging findings in temporal lobe epilepsies.

Handb Clin Neurol

August 2022

AP-HP, Department of Neurology and Department of Clinical Neurophysiology, Epilepsy and EEG Unit, Reference Center for Rare Epilepsies, Pitié-Salpêtrière Hospital, Paris, France; Sorbonne Université, Paris Brain Institute, Team "Dynamics of Neuronal Networks and Neuronal Excitability", Paris, France. Electronic address:

Temporal lobe epilepsy (TLE) is the most common type of focal epilepsy. First descriptions of TLE date back in time and detailed portraits of epileptic seizures of temporal origin can be found in early medical reports as well as in the works of various artists and dramatists. Depending on the seizure onset zone, several subtypes of TLE have been identified, each one associated with peculiar ictal semiology.

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Cohort study of infantile epileptic spasms syndrome: etiological analysis and treatment of corticosteroids.

Seizure

October 2022

Ministry of Education Key Laboratory of Child Development and Disorders, International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Pediatrics, National Clinical Research Center for Child Health and Disorders, Chongqing, China; Department of Neurology, CHCMU, Chongqing, China.

Background: Infantile epileptic spasms syndrome (IESS) is the most common type of severe epilepsy in infants. However, etiological frequency and optimized therapy, particularly corticosteroid regimen and dose, remain unknown.

Methods: An ambispective study of an IESS-diagnosed cohort was conducted.

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Cognition, adaptive skills and epilepsy disability/severity in patients with Lennox-Gastaut syndrome undergoing deep brain stimulation for epilepsy in the ESTEL trial.

Seizure

October 2022

Department of Medicine (Austin Health), University of Melbourne, Heidelberg VIC 3084, Australia; Department of Neurology, Austin Health, Heidelberg, VIC 3084, Australia; The Florey Institute of Neuroscience and Mental Health, Heidelberg, VIC 3084, Australia; Murdoch Children's Research Institute, Parkville, VIC 3052, Australia.

Purpose: We previously reported seizure and EEG outcomes of the ESTEL study (Electrical Stimulation of Thalamus for Epilepsy of Lennox-Gastaut phenotype). To assess potential cognitive and behavioral changes during chronic, duty-cycle stimulation of bilateral thalamic centromedian nucleus, we compared standardized cognitive and behavioral measurements, as well as caregiver assessments of disability/severity, before implantation and after 3-months stimulation.

Methods: Twenty patients with LGS (17-37 years;13 females) were studied; one participant was not randomized due to DBS device removal, with outcomes of 19 remaining participants reported here.

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Objective: Temporal lobe epilepsy (TLE) and depression are common comorbid disorders whose underlying shared neural network has yet to be determined. Although animal studies demonstrate a role for the dorsal bed nucleus of the stria terminalis (dBNST) in both seizures and depression, and human clinical studies demonstrate a therapeutic effect of stimulating this region on treatment-resistant depression, the role of the dBNST in depressed and nondepressed TLE patients is still unclear. Here, we tested the hypothesis that this structure is morphologically abnormal in these epilepsy patients, with an increased abnormality in TLE patients with comorbid depression.

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The amygdala is one of the most common origins of seizures, and the amygdala mouse model is essential for the illustration of epilepsy. However, few studies have described the experimental protocol in detail. This paper illustrates the whole process of amygdala electrical kindling epilepsy model making, with the introduction of a method of bipolar electrode fabrication.

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Astrocytic GABA transporter 1 deficit in novel SLC6A1 variants mediated epilepsy: Connected from protein destabilization to seizures in mice and humans.

Neurobiol Dis

October 2022

Department of Neurology, Vanderbilt University Medical Center, USA; Department of Pharmacology, Vanderbilt University, USA; Vanderbilt Kennedy Center of Human Development, University Medical Center, Nashville, TN, 37232, USA. Electronic address:

Objective: Mutations in γ-aminobutyric acid (GABA) transporter 1 (GAT-1)-encoding SLC6A1 have been associated with myoclonic atonic epilepsy and other phenotypes. We determined the patho-mechanisms of the mutant GAT-1, in order to identify treatment targets.

Methods: We conducted whole-exome sequencing of patients with myoclonic atonic epilepsy (MAE) and characterized the seizure phenotypes and EEG patterns.

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